Long term survival after extended resection of primary atrial myxosarcoma

D. Kakaty, M. Grapow, A. Tzankov, A. Hiebinger, M. Wiese, F. Eckstein, D. Lardinois

Objective

Primary cardiac sarcomas are rare and generally associated with a poor prognosis with a median overall survival of 17 months. The identification of features that distinguish a cardiac sarcoma from a benign myxoma is difficult both clinically and histologically.

Methods

A 48-year-old female presented with progressive dyspnea, weakness and loss of weight. A giant left atrial tumor was discovered. The patient underwent resection of the atrial septum including the atrial roof with subsequent reconstruction of the left atrium and the left upper pulmonary vein. Histopathological examination revealed left atrial myxoma and thus no further adjuvant therapy was performed. Followup imaging with PET-CT at 14 months revealed evidence of local recurrence. An open biopsy was performed, revealing myxosarcoma. A new analysis of the initially resected tumour confirmed the diagnosis of myxosarcoma. Following 4 cycles of chemotherapy, the patient underwent an extended reoperation with complete resection of the tumour mass including a left pneumonectomy due to massive infiltration of the hilus. Recovery was uneventful. Subsequent follow-up at 4 years shows no radiological evidence of any further recurrence. The patient is satisfied with good quality of life.

Results

Histopathological diagnosis of primary myxosarcoma can be challenging. Therapy usually consists of an induction treatment followed by complete resection if possible. Despite such an aggressive therapy concept, survival often remains poor.

Conclusion

In our case, we were able to demonstrate that with an aggressive therapy and an interdisciplinary approach, a long term disease free survival can be achieved.